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Epstein Barr virus (EBV) related lymphoproliferative diseases may occur in immunocompromised patients or patients with a history of drug use causing immunodeficiency. EBV positive mucocutaneous ulceration in the new classification of lymphoproliferative diseases in 2016 is very rare in children. Involvement occurs in the skin, oral mucosa, and gastrointestinal system. Gastric involvement is very rare in the literature. There is no case of gastric involvement in children. There are no specified modalities in the treatment of EBV positive mucocutaneous ulceration. We presented our pediatric patient with ataxia telangiectasia who presented with abdominal pain and difficulty swallowing and diagnosed with EBV positive mucocutaneous ulceration in the stomach. We started brentuximab vedotin during the treatment process, and complete remission was achieved after 6 cures of treatment. Our patient is the first case of EBV positive mucocutaneous ulceration in the pediatric case series., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Pulmonary fibrosis caused by bleomycin-induced pneumonia (BIP) is the most important side effect limiting the use of bleomycin and is mainly treated with corticosteroids. However, 1% to 4% of patients do not respond to corticosteroid therapy. Idiopathic pulmonary fibrosis and BIP develop by similar pathophysiological mechanisms. Nintedanib is a tyrosine kinase inhibitor used successfully in the treatment of idiopathic pulmonary fibrosis and there is no information about its use in BIP treatment. Here, we would like to present a 13-year-old boy with Hodgkin lymphoma who developed BIP after 2 cycles of ABVD (Adriamycin, bleomycin, vinblastine, and dacarbazine) and 4 cycles of BAECOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone), whose respiratory failure impaired despite corticosteroid therapy, but was successfully treated with nintedanib., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Constitutional mismatch repair deficiency (CMMRD) is an autosomal recessively inherited childhood cancer predisposition syndrome results from biallelic germline mutations affecting the key DNA mismatch repair gene: MLH1, MSH2, MSH6, or PMS2. CMMRD is associated with a high risk of developing early onset of central nervous system tumors, hematologic, and intestinal tract tumors. Clinical manifestations, genetic screening, and cancer prevention strategies are limited. In this report we present a patient with metachronous Wilms tumor, glioblastoma, and acute T-cell lymphoblastic leukemia. He had cutaneous features of neurofibromatosis type 1 (NF1). Molecular testing revealed a novel homozygous mutation in MSH6 (c.2590G>T; p.G864*) that has not been reported previously. CMMRD should be considered in patients with cutaneous features similar to NF1 if tumor is found other than expected tumors in NF, early onset cancer, and strong family history of cancer., Competing Interests: The authors declare no conflict of interest., (Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.)

Erdheim-Chester disease (ECD) is a proliferative disorder of non-Langerhans histiocytes with a higher incidence in the fifth to seventh decades and rarer occurrence in the pediatric population. Although ECD typically involves bone, it can also affect the central nervous system, cardiovascular system, retro-orbital space, retroperitoneal space, and kidneys, lungs, and skin. A 13-year-old Syrian girl who presented with multisystemic involvement was diagnosed with ECD. The B-Raf proto-oncogene V600E mutation was not detected in ECD lesions. Response to the high-dose interferon-α therapy was excellent in this pediatric patient. In this article, pediatric ECD case reports are also reviewed.

Mucoepidermoid carcinoma arising in Warthin tumor of the parotid gland is an extremely rare entity. This is so far described only in the adult age group, and only one patient has been reported in the pediatric age group until today. Herein, we describe our patient and review the literature. This was a case of a 9-year-old girl admitted with a painless swelling in the left side of her face for at least 2 weeks. Histopathologic examination of total parotidectomy specimens revealed mucoepidermoid carcinoma arising in the Warthin tumor. This case is the youngest patient reported to date.

Gastrinoma is a hormone-secreting tumor associated with the Zollinger-Ellison syndrome. It is quite rare among children. The discovery of gastrinomas in unusual locations such as lymph nodes, bones, ovaries, and the liver poses a diagnostic dilemma as to whether the tumor is primary or metastatic. Here, we present a case of a primary gastrinoma within a lymph node.

Background: Despite the presence of reports on correlation between major congenital defects and cancer, very few studies have investigated the frequency of minor anomalies in childhood malignancy. The aim of this study was to determine the prevalence of minor anomalies in children with lymphoma and solid tumors., Procedure: A total of 281 well-defined minor anomalies were determined in 116 patients. The patients were compared with age-matched and sex-matched healthy control subjects., Results: Concerning the cumulative data, 87.9% (102/116) of the patients and 37.9% (44/116) of the controls had at least 1 minor anomaly (P < 0.05). A total of 281 minor anomalies (2.42 per subject) were noted in the patient group, whereas 41 (0.35 per subject) were noted in the control group (P < 0.05). The eye, ear, mouth, hand, and feet anomalies were statistically higher in the patient group than the control group (P < 0.05)., Conclusions: Our results contribute to the understanding of the role of genetic factors in childhood cancer. Future studies might be directed toward identifying the developmental pathways and the relevant genes that are involved in relation to childhood cancer and minor anomalies.

Primary hepatic non-Hodgkin lymphoma is extremely rare in childhood and can be overlooked in differential diagnosis of liver masses. In this study, we describe an unusual case of primary hepatic Burkitt lymphoma in childhood.

Background: Brucellosis continues to be an important cause of fever in underdeveloped countries and in the rural areas of developed world. It is a multisystemic disease, associated with a wide variety of symptoms. A wide variety of symptoms, including hematologic abnormalities, such as anemia, thrombocytopenia, pancytopenia, dissemine intravascular coagulation, and leucopenia could be seen. The aim of the study is to review the hematologic findings of brucellosis in childhood., Procedure: In this short study, the records of 146 children with brucellosis were evaluated for hematologic manifestation retrospectively. Among them, 9 patients had pancytopenia and 5 had brucella-induced immune thrombocytopenia and were identified in a 5-year period between June 2004 and July 2009., Results: Eight of the 9 patients with pancytopenia had Brucella melitensis isolated from blood cultures and/or bone marrow cultures, and all 9 patients had Brucella agglutination titers of at least 1:320. All patients with immune thrombocytopenia blood cultures were positive for Brucella. Except 1 patient the pancytopenia in these patients regressed completely and their peripheral blood counts returned to normal after treatment of Brucella infection. One patient was not responding to the brucella treatment and underwent allogeneic hematopoietic stem cell transplantation. All patients with brucella-induced immune thrombocytic purpura were symptomatic and had severe thrombocytopenia, they were placed on intravenous gamma globulin for 2 days. Between day 3 and day 5 platelet counts increased in these patients., Conclusion: Brucellosis should be considered as a possible diagnosis among patients with pancytopenia and immune thrombocytopenic purpura in endemic regions.

Vincristine commonly induces peripheral neuropathy but rarely causes voiding dysfunction. In this report, we describe a case of neurogenic bladder and peripheral neuropathy caused by the neurotoxic effect of vincristine and documented by urodynamic testing and electromyography. Neurologic evaluation aided in monitoring and understanding this problem.